Antenatal immunoglobulin for prevention of neonatal hemochromatosis

Neonatal hemochromatosis (NH) is a rare disease with a poor prognosis, particularly prior to 2008. Antenatal maternal high‐dose immunoglobulin (Ig) is effective in preventing NH recurrence, but the adverse effects of this treatment have not been documented as yet. Here, we report on a patient who underwent high‐dose Ig treatment to prevent NH recurrence. The patient was a 31‐year‐old pregnant Japanese woman. Her first child died of NH after receiving living donor liver transplantation. The patient received high‐dose Ig treatment to prevent recurrence of NH from gestational weeks 16 to 35. During the treatment, platelet count gradually decreased, and cesarean section was required at 35 gestational weeks. The child did not develop liver failure. High‐dose Ig prevented the recurrence of NH. Caution should be exercised due to possible adverse effects of this treatment.     

Use of oxygen for delivery room neonatal resuscitation in non-tertiary Australian and New Zealand hospitals: A survey of current practices, opinions and equipment

Background: Delivery room resuscitation of hypoxic newborn infants with pure or 100% oxygen causes oxidative toxicity and increases mortality. Current international resuscitation guidelines therefore recommend that oxygen be used judiciously. However, this requires staff education and special equipment that may not be available in non-tertiary maternity hospitals where the majority of births occur. Aim: To determine current attitudes, practices and available equipment for the use of air and blended oxygen for newborn delivery room resuscitation in non-tertiary maternity hospitals of Australia and New Zealand (ANZ). Methods: Structured questionnaires sent by mail and e-mail after personal phone contact. A total of 203 eligible hospitals in ANZ were identified. A second mailing was conducted a month later for non-responders. Responders: Final response rate was 64% (n= 130: 70% physicians, 30% midwives). The majority (121, 93%) of respondents were aware of Australian Resuscitation Council recommendations, but only one in five hospitals had the capacity to deliver blended oxygen and 38% used pulse oximeters at delivery. Only 24 (18.5%) hospitals had guidelines. Air would be used by 68 (57%) hospitals to resuscitate term infants compared to 35 (31%) for preterm infants. Most (111, 91%) advocated the use of blended oxygen despite the lack of facilities. Conclusion: Only one in five ANZ non-tertiary maternity hospitals had the capacity to resuscitate newborn infants with air or blended oxygen. Most are aware of current recommendations and agreed that the use of less oxygen would be beneficial for this purpose. Further study into the necessary infrastructure required to implement these guidelines are recommended.     

Physician counselling practices and decision-making for extremely preterm infants in the Pacific Rim

OBJECTIVES: This study was undertaken to evaluate physician counselling practices and resuscitation decisions for extremely preterm infants in countries of the Pacific Rim. We sought to determine the degree to which physician beliefs, parents' opinion and medical resources influence decision-making for infants at the margin of viability. METHODS: A survey was administered to neonatologists and paediatricians who attend deliveries of preterm infants in Australia, Hong Kong, Japan, Malaysia, Taiwan and Singapore. Questions were asked regarding physician counselling practices, decision-making for extremely preterm infants and demographic information. RESULTS: Physicians counsel parents antenatally with increasing frequency as gestational age increases. Most physicians discuss infant mortality and morbidity with parents prior to delivery. Physicians less frequently discuss the option of no resuscitation of an extremely preterm infant, withdrawal of support at a later time, or financial costs to parents. Severe congenital malformations, perception of a poor future quality of life, parental wishes and a high probability of death for the infant are influential in limiting resuscitation in very preterm infants for a majority of physicians. Less influential factors are parent socioeconomic status, language barriers, financial costs for the family, allocation of national resources, moral or religious considerations, or fear of litigation. Physician thresholds for resuscitation of infants ranged between 22 and 25 weeks gestation and between 400 and 700 g birthweight. CONCLUSIONS: We report physician beliefs and practices regarding resuscitation and the counselling of parents of extremely preterm infants in Pacific Rim countries. While we find variation among countries, physician practices appear to be determined by ethical decision-making and medical factors rather than social or economic factors in each country.     

Pediatric liver transplantation for neonatal‐onset Niemann‐Pick disease type C: Japanese multicenter experience

Niemann‐Pick disease type C (NPC) is a rare autosomal recessive inherited disease characterized by lysosomal accumulation of free cholesterol in macrophages within multiple organs. Infantile‐onset NPC often presents with jaundice and hepatosplenomegaly from birth, but these symptoms usually improve during early childhood, and it rarely progresses to liver failure. We report three cases from different hospitals in Japan; the patients developed neonatal‐onset NPC, and liver transplantation (LT) was performed as a life‐saving procedure. LT was performed at 19 days, 59 days, and 4 months of age, respectively. The last patient was diagnosed with NPC before LT, while the first two patients were diagnosed with neonatal hemochromatosis at LT. In these two patients, the diagnosis of NPC was made more than a year after LT. Even though oral administration of miglustat was started soon after the diagnosis of NPC, all patients showed neurological regression and required artificial respiratory support. All patients survived more than one year after LT; however, one patient died due to tracheal hemorrhage at 4.5 years of age, and another one patient was suspected as recurrence of NPC in liver graft. In conclusion, while LT may be a temporary life‐saving measure in patients with neonatal‐onset NPC leading to liver failure, the outcome is poor especially due to neurological symptoms. A preoperative diagnosis is thus critical.     

Summary of Japanese Neonatal Cardiopulmonary Resuscitation Guidelines 2015

The Japan Resuscitation Council joined the International Liaison Committee on Resuscitation (ILCOR) as a member of the Resuscitation Council of Asia in 2006. In 2007, the Japan Society of Perinatal and Neonatal Medicine (JSPNM), which is a member of an affiliated body, launched the Neonatal Cardiopulmonary Resuscitation (NCPR) program as an authorized project to ensure that all staff involved in perinatal and neonatal medicine can learn and practice neonatal cardiopulmonary resuscitation based on the Consensus on Science with Treatment Recommendations developed by ILCOR. The content of courses in the NCPR program is based on the NCPR guidelines. These guidelines are revised by the Japan Resuscitation Council according to the Consensus on Science with Treatment Recommendations, which is updated by ILCOR every 5 years. The latest updated edition in Japanese was published in 2016 and we translated these Japanese guidelines to English in 2018. Here, we introduce a summary of the NCPR guidelines 2015 in Japan. The NCPR 2015 algorithm has two flows, “lifesaving flow” and “stabilization of breathing flow” at the first branching point after the initial step of resuscitation.     

Congenital amegakaryocytic thrombocytopenia

Congenital amegakaryocytic thrombocytopenia (CAMT) is clinically characterized by thrombocytopenia presenting at birth in a child without congenital or skeletal malformations, reduced or absent bone marrow megakaryocytes, and eventual progression to bone marrow failure. Molecular studies in most cases confirm homozygous or compound heterozygous mutations in the thrombopoietin receptor c-Mpl. In addition to the clinical importance of recognizing this disorder, characterization of mutations identified in patients with CAMT has led to insights into thrombopoietin receptor structure and function. This review will summarize the diagnosis, pathophysiology, and management of CAMT.     

Laparotomy for necrotizing enterocolitis: Intensive care nursery compared with operating theatre

OBJECTIVE: To determine whether neonates requiring laparotomy for necrotizing enterocolitis (NEC) are more stable perioperatively and have less disruption of physiological parameters if surgery is performed in the neonatal intensive care unit (NICU) compared with the operating theatre (OR). METHODOLOGY: A retrospective case review was performed on 233 neonates referred for further surgical management of severe NEC in the period January 1989 to December 1997. Mortality and morbidity were compared by calculating the score for neonatal acute physiology (SNAP) and its attendant risk of mortality score. Thirty-six separate physiological variables were also compared pre- and postoperatively and the mean postoperative change was calculated. RESULTS: For neonates weighing less than 1500 g, mortality was linked to illness severity, as measured by SNAP, rather than operative location. Specific adverse events associated with secondary transfer to the OR included hypothermia, deterioration in oxygenation parameters, ventilation parameters and platelet count. The liberal use of blood products, albumin and bicarbonate in perioperative resuscitation may have obscured other effects. CONCLUSIONS: The use of the neonatal intensive care nursery for surgery on neonates weighing less than 1500 g with severe NEC can be justified and such use should be encouraged. In contrast, secondary transport of neonates weighing less than 1500 g to the OR for laparotomy is associated with significant deterioration in a number of physiological parameters, which may impact on morbidity.     

Neonatal cholestasis and glucose‐6‐P‐dehydrogenase deficiency

We report a Caucasian neonate with chronic non‐spherocytic hemolytic anemia due to a class I G6PD deficiency. A novel mutation missense mutation in exon eight of the G6PD gene was detected (c.827C>T p.Pro276Leu). Bilirubin peaked on day 5 at 24 mg/dl with a conjugated bilirubin of 17 mg/dl. Jaundice resolved within 4 weeks. A detailed work‐up failed to reveal other specific factors contributing to cholestasis. Severe hemolytic disease of the newborn may cause cholestasis even in the absence of associated primary hepato‐biliary disease. Pediatr Blood Cancer 2010;54:758–760. © 2010 Wiley‐Liss, Inc.     

Nasal continuous positive airway pressure for neonatal back transfer

We report the use of nasal continuous positive airway pressure (nCPAP) for neonatal back transfer of 51 babies. nCPAP may be used to provide safe respiratory support during back transportation of infants. Babies transferred on nCPAP were significantly smaller and of younger postmenstrual age than historical comparators who had to wait for transfer until independent of nCPAP. nCPAP transferred babies spent significantly less time on the intensive care unit.     

Neonatal sepsis and infection control policies in Australia

The pattern of neonatal bacterial infection, its management and the types of infection control policy were ascertained in 20 neonatal intensive care units (NICU) in Australia by questionnaire survey. Group B Streptococcus and Escherichia coli were the predominant organisms responsible for perinatally acquired infection for which the most common antibiotic combination used was Penicillin and Gentamicin. Staphylococcus epidermidis and aureus (majority Methicillin resistant) were the predominant organisms responsible for nosocomial infection for which the most common antibiotic combination used was Vancomycin and Cefotaxime. A Serratia epidemic was experienced in four NICU. Lumbar and suprapubic punctures were frequently done as part of the diagnostic workup in nosocomial infections but not with perinatally acquired infections. Haematological indices considered useful as a diagnosis aid varied between NICU and acute phase reactants were rarely relied upon for diagnosis or for monitoring treatment response. Granulocyte transfusion and intravenous immunoglobulins were infrequently used in therapy. No consensus was found on infection control policies. Eight NICU required routine gowning on entry, two restricted sibling visiting and four restricted visiting by relatives and friends. Although routine bacteriological surveillance on staff or equipment was uncommon, serial cultures were carried out in infants from the body surface in six NICU and from the endotracheal aspirate in 17 NICU. Antiseptics used with technical procedures included iodine, chlorhexidine and alcohol used singly or in combination. Skin and umbilical cord care also varied between NICU. The literature was reviewed to determine the effectiveness or otherwise of some of the existing policies in the prevention or management of neonatal infection to encourage consensus towards a more rational approach to neonatal infection in Australia.     

Angiography and the conservative management of neonatal adrenal haemorrhage

Neonatal angiography provided an additional criterion in the diagnosis of unilateral neonatal adrenal haemorrhage. Non-operative management was thus enabled. It is suggested that the prevalent operative approach to all neonatal perirenal masses should be revised.     

Management of phototherapy for neonatal hyperbilirubinemia: Is a new radiometer applicable for all wavelengths and light source types?

Background: To evaluate the clinical effects of phototherapy for neonatal hyperbilirubinemia, it is necessary to measure the rate of cyclobilirubin production, which represents the main photochemical pathway of bilirubin metabolism. Since the Atom Phototherapy Analyzer can be used to calculate the theoretical relative light energy of irradiance as a means of assessing the cyclobilirubin production rate for each wavelength spectrum, the clinical effect of phototherapy can be evaluated regardless of the light source type. Using the Atom Phototherapy Analyzer, the correlation between the irradiance of various light sources with different peak wavelengths and the rate of cyclobilirubin production was investigated in vitro. We also investigated the utility of green LED in vitro. Methods: A bilirubin‐albumin complex solution was prepared, poured into tubes, and irradiated using various light sources. All light sources used were bed‐type phototherapy devices; that is, green and blue LED and green and blue fluorescence tubes. The concentrations of photoisomers were measured after irradiation and compared with the irradiance of the light sources. Results: The irradiance measured by the Atom Phototherapy Analyzer decreased in the following order: blue fluorescence tube > green LED > blue LED > green fluorescence tube. The cyclobilirubin production rates and irradiance values of the light sources were significantly positively correlated (R²= 0.93, P < 0.05). Conclusion: Our data indicate that the Atom Phototherapy Analyzer can be used to objectively evaluate the effects of phototherapy using various light sources. Further, the effects of green LED were similar to those of other light sources in vitro.     

Neonatal screening for congenital hypothyroidism and phenylketonuria in China

Background  Neonatal screening is helpful to prevent serious disabitily and sufferings caused by congenital or inherited disease. This study was to review the status of neonatal screening for congenital hypothyroidism (CH) and phenylketonuria (PKU) in China. Methods  We analyzed data of neonatal screening for CH and PKU in the past two decades which were obtained from the national network of neonatal screening centers collected by the National Center for Clinical Laboratory. Results  Of 18.8 million newborns screened from 1985 to 2007, 9198 were identified with CH, giving a prevalence of 1/2047. In 19.0 million newborns screened in the same period, 1638 had PKU, with a prevalence of 1/11 572. An increasing number of neonates have been subjected to neonatal screening in China annually during this period. Data from Zhejiang Neonatal Screening Center showed that the recall rate of neonates suspected with CH and PKU was 95.52% in 2007. Confirmatory tests were performed and treatments were initiated in most of the neonates with CH and PKU within a month after birth. Conclusions  More governmental support at different levels is needed to make neonatal screening more efficient. The screening should be improved with a satisfactory control system including shorter time of report and a higher recall rate.